JoNel Aleccia NBC News
Oct. 2, 2013 at 8:56 AM ET
When
a neurosurgery patient died from a rare and fatal brain disorder at a
New Hampshire hospital this summer, it sent a ripple of panic across the
region, particularly for
15 people warned
that they may have been exposed to Creutzfeldt-Jakob disease through
contaminated hospital surgical equipment. Everyone wondered who might
spread this dire bug, and who might be at risk.
But families of
others who have suffered — and died — from the little-known but
invariably deadly disease say that reaction is only the beginning of the
fear, discrimination and rejection that CJD patients face.
Courtesy Karen Bailey
Jerry
Bailey, 64, lies in a Utah hospital in August, surrounded by his
daughters, Amanda Bailey, Raelynn Miller, Nichole Bailey and Stephanie
Rathbone. The Boise, Idaho, man died five weeks later of
Creutzfeldt-Jakob disease, a rare and fatal brain disorder. His body was
rejected by four funeral homes and a crematorium out of fear of
infection, family members said.
Across the country,
funeral homes and crematoriums are routinely refusing to accept the
bodies of CJD patients out of fear of infection, despite health
guidelines that say that — with standard precautions — embalming and
burial is perfectly safe.
“It happens a lot,” said Robert Kassai, a
New Jersey funeral director who also sits on the board of the CJD
Foundation, an advocacy group. “There are many, many instances where
families call me saying they’ve been turned away.”
Some families
report that their loved ones who died from CJD were removed from the
hospital, placed in double body bags and taken directly to a crematorium
with no warning. Others tell NBC News that funeral workers forced
pallbearers to wear medical gloves and told mourners to stand far back
from the gravesite and to disperse quickly after the ceremony.
“The
funeral issue is the bane of our existence, basically,” said Florence
Kranitz, president of the CJD Foundation. “They get people at their most
vulnerable, when they’ve been hit by the freight train of this
disease.”
CJD is very rare — with about one death per million
people worldwide each year. But Kassai estimates that dozens of the
estimated 300 people in the U.S. who annually die from the degenerative
brain disease are rejected by the people in charge of final disposition.
That
was the case for Amanda Bailey of Ogden, Utah, whose 64-year-old
father, Jerry Bailey, died Sept. 18, just four months after exhibiting
the first signs of CJD. Typical symptoms include memory and cognitive
problems, vision problems and, later, trouble walking and speaking.
After
his devastating decline, Jerry Bailey’s remains were rejected by four
Salt Lake City funeral homes and a crematorium, she said.
“They
wouldn’t even cremate him because they worried that the spores would
become airborne,” said Bailey, 27. “They apologized and said they were
sorry, but they didn’t want to risk the health and the safety of the
people there.”
Never mind that CJD is caused not by spores but by
prions, virus-like proteins that manage to transform other proteins into
disease-causing shapes. Like most patients, Jerry Bailey had sporadic
CJD, which occurs spontaneously, with no known cause or cure. It’s not
the variant form of the disease that causes a human form of “mad cow
disease,” associated with eating beef.
And never mind that
infectious disease experts say that the prions, though hard to kill,
don’t spread easily and not through the air or through ordinary skin
contact. It takes injection, transplantation or even consumption of the
tainted tissue to cause infection.
“’There’s no known actual risk.
Morticians are in no more danger at all than anyone else,” said Dr.
Richard T. Johnson, a CJD expert and a neurology professor at Johns
Hopkins Medicine, in Baltimore, Md.
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Fatal rare brain disease confirmed in N.H. patient; 15 possibly exposed
Sep. 21, 2013 at 1:04 AM ET
A
neurosurgery patient treated at a New Hampshire hospital this spring
did have a rare brain disorder known as Creutzfeldt-Jakob Disease,
health officials confirmed Friday. That means that 15 other people in
three states may have been exposed to the invariably deadly infection
through potentially tainted surgical equipment.
Autopsy results
came back positive for CJD from the National Prion Disease Pathology
Surveillance Center and were reported to the New Hampshire Department of
Health and Human Services and Catholic Medical Center, where the
surgery took place.
Earlier this month, New Hampshire officials
notified eight patients who may have been exposed to CJD through shared
equipment. Five others in Massachusetts and two in Connecticut were also
warned of the risk, health officials in those states said.
"Though
we are not surprised by the test results, we are saddened by the toll
this disease takes on families and our sympathies go out to all those
affected," said Dr. Jose Montero, New Hampshire's director of public
health, in a statement. There is no way to confirm the disease except
through autopsy after a patient's death.
The initial patient
turned out to have sporadic CJD, which occurs spontaneously. It's not
the variant form of the disease that causes a human type of "mad cow
disease" and is associated with eating beef contaminated with the cattle
version of the infection, called bovine spongiform encephalotpathy, or
BSE, experts said.
The problem arose because standard hospital
disinfection techniques cannot eradicate the prion that causes CJD. A
prion is a protein and the type that causes BSE and CJD is misfolded and
somehow manages to transform other proteins into disease-causing shape.
The
initial patient had surgery at Catholic Medical Center in Manchester,
N.H., where eight others were also treated. The five Massachusetts
patients underwent surgery at Cape Cod Hospital in Hyannis, while the
two Connecticut patients were treated at the Veterans Affairs Hospital
in West Haven, health officials said.
Read More Here
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Maine Gov. Paul LePage in June 2013. (Robert F. Bukaty/AP)
